Rare Rheumatology News

Disease Profile

Fox-Fordyce disease

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Miliaria, apocrine; Apocrine miliaria; Fox-Fordyce syndrome

Categories

Skin Diseases

Summary

Fox-Fordyce disease is a chronic skin disease most common in women aged 13-35 years.[1] It is characterized by the development of intense itching in the underarm area, the pubic area, and around the nipple of the breast as a result of perspiration which becomes trapped in the sweat gland and surrounding areas.[2] The cause is unknown, but heat, humidity, and stress may play a role. Treatment may include the use of retinoids, antibiotics, and immunosuppressants.[1][3]

Treatment

Individuals with Fox-Fordyce disease should consult with a dermatologist regarding treatment. Some of the recent treatments for the FFD include: interlesional glucocorticoids, topical steroids, oral and topical retinoids, topical clindamycin, topical pimecrolimus cream, benzoyl peroxide, oral antibiotics and contraceptives or antiandrogenic hormonal therapy.[4][3] Pimecrolimus and tacrolimus, both with significant anti-inflammatory activity and low side-effects, have provided rapid improvement in a limited number of cases.[1] Other forms of treatment used are ultraviolet radiation (phototherapy), dermabrasion, liposuction and surgical excision.[4] Immunosuppressants have been utilized with modest success. For individuals who do not respond to medications, destruction or removal of the apocrine sweat glands (glands that surround hair follicles) has been effective in some cases. [4]

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Fox-Fordyce disease. Click on the link to view a sample search on this topic.

References

  1. Christopher R Gorman. Fox-Fordyce Disease. Medscape. Sep 23, 2015; https://emedicine.medscape.com/article/1070560-overview.
  2. Timothy H. McCalmont. Fox Fordyce Disease. National Organization for Rare Disorders (NORD). 2010; https://rarediseases.org/rare-diseases/fox-fordyce-disease/.
  3. Vanessa Ngan. Fox-fordyce disease. DermNet New Zealand. 2005; https://dermnetnz.org/topics/fox-fordyce-disease/.
  4. Yost, J., Robinson, M., & Meehan, S. A. Fox-Fordyce disease. Dermatology online journal. 2012; 18(12):https://escholarship.org/uc/item/6km4c88v. Accessed 7/31/2014.

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